Successful treatment of severe hemophagocytic lymphohistiocytosis caused by chronic active Epstein-Barr virus infection with emergent PBSCT

Abstract

We report the case of an 8-year-old boy with hemophagocytic lymphohistiocytosis (HLH) caused by chronic active EBV infection (CAEBV). He was transferred to our hospital because he was refractory to steroids and multidrug chemotherapy. Although we treated him with intensified multidrug chemotherapy, his condition deteriorated. The patient underwent emergent peripheral blood stem cell transplantation (PBSCT) from his mother. After the conditioning regimen, his high fever and laboratory findings resolved. He achieved complete donor engraftment on day 18 and complete remission with an EBV-DNA load that was lower than the detection limit. If a case of HLH caused by CAEBV is refractory to steroids or multidrug chemotherapy, we need to carry out hematopoietic stem cell transplantation immediately.